The Ubiquitin-Proteasome System in Huntington’s Disease
2005; SAGE Publishing; Volume: 11; Issue: 6 Linguagem: Inglês
10.1177/1073858405280639
ISSN1089-4098
AutoresAlberto Valera, Miguel Díaz‐Hernández, Félix Hernández, Zaira Ortega, José J. Lucas,
Tópico(s)Mitochondrial Function and Pathology
ResumoThe main histopathological feature of Huntington's disease (HD) is the presence of protein aggregates that are gathered into inclusion bodies. So far the mechanisms that lead to inclusion formation as well as their role in the pathogenesis of HD are not totally understood. However, it is well established that inclusion bodies contain components of the ubiquitin-proteasome system. Accordingly, it has been postulated that impairment of this machinery can be one of the causes of this disorder. In this review, the authors summarize the state of current knowledge about this hypothesis.
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